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As humans, or indeed as any mammal, bird, or insect, we contain a specific molecular makeup that is driven by vast numbers of these miniscule powerhouses residing in most of our cells (mature red blood cells notwithstanding), quietly replicating, living independent lives and containing their own DNA. Everything we do, from running a marathon to breathing, is driven by these small batteries, and yet there is evidence that these molecular energy sources were originally bacteria, possibly parasitic, incorporated into our cells through symbiosis. Dysfunctions in these organelles can lead to debilitating, and sometimes fatal, diseases of almost all the bodies\u2019 major organs. Mitochondrial dysfunction has been implicated in a wide variety of human disorders either as a primary cause or as a secondary consequence. To better understand the role of mitochondrial dysfunction in human disease, a multitude of pharmacologically induced and genetically manipulated animal models have been developed showing to a greater or lesser extent the clinical symptoms observed in patients with known and unknown causes of the disease. This review will focus on diseases of the brain and spinal cord in which mitochondrial dysfunction has been proven or is suspected and on animal models that are currently used to study the etiology, pathogenesis and treatment of these diseases.<\/jats:p>","DOI":"10.1007\/s10545-011-9375-8","type":"journal-article","created":{"date-parts":[[2011,7,13]],"date-time":"2011-07-13T13:21:33Z","timestamp":1310563293000},"page":"193-210","source":"Crossref","is-referenced-by-count":28,"title":["Modeling mitochondrial dysfunctions in the brain: from mice to men"],"prefix":"10.1002","volume":"35","author":[{"given":"Megan E.","family":"Breuer","sequence":"first","affiliation":[{"name":"Department of Biochemistry, Nijmegen Centre for Molecular Life Sciences Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"},{"name":"Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"},{"name":"Centre for Systems Biology and Biochemistry, Nijmegen Centre for Molecular Life Sciences Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"},{"name":"Department of Biochemistry, Nijmegen Centre for Mitochondrial Diseases Radboud University Nijmegen Medical Centre P.O. 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